Original Article

Low incidence of alpha-1-antitrypsin deficiency in Iranian patients with neonatal cholestasis

Volume 26 · Issue 3 Publish Date: May 15, 2015
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Farzaneh Motamed
Department of Pediatrics, Pediatrics Center of Excellence, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
Sanaz Mehrabani
Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran
Maryam Monajemzadeh
Department of Pediatrics, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Mohammad Taghi Haghi Ashtiani
Department of Pediatrics, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Sima Hosseinverdi
Research Center for Immunodeficiencies, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Masoud Houshmand
Department of Medical Genetics, Special Medical Center, Tehran, Iran
Omid Aryani
Department of Medical Genetics, Special Medical Center, Tehran, Iran
Mehri Najafi
Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Fatemeh Farahmand
Department of Pediatrics, Pediatrics Center of Excellence, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
Mohammad Ali Kiani
Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Ahmad Khodadad
Department of Pediatrics, Pediatrics Center of Excellence, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
Gholam Hossein Fallahi
Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran
Gholamreza Khatami
Department of Pediatrics, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Nima Rezaei
Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Motamed, F., Mehrabani, S., Monajemzadeh, M., Ashtiani, M. T. H., Hosseinverdi, S., Houshmand, M., … Rezaei, N. (2015). Low incidence of alpha-1-antitrypsin deficiency in Iranian patients with neonatal cholestasis. Turkish Journal of Gastroenterology, 26(3), 251–253. https://doi.org/10.5152/tjg.2015.6339
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Abstract

Abstract Background/Aims: There is little data concerning the incidence of alpha-1-antitrypsin”(AAT) deficiency, the most common genetic cause of liver disease, among children with neonatal cholestasis in Iran. Thus, this study was performed to analyze AAT deficiency in this group of patients. Materials and Methods: DNA samples from patients with neonatal cholestasis were investigated for Pi S and Pi Z alleles, using polymerase chain reaction–restriction fragment length polymorphism. Results: Thirty patients with neonatal cholestasis were enrolled. Among those who underwent biopsies, the results revealed neonatal hepatitis in 19, bile duct paucity in 1, steatohepatitis in 1, bile duct proliferation in 1, cirrhosis in 2, fibrosis in 2, and extrahepatic biliary atresia in 1 patient. No mutant allele was found in any patient. Conclusion: The incidence of AAT deficiency is very low in Iran; therefore, screening for AAT is not recommended for patients with neonatal cholestasis in Iran.

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Article Info
Published In
Journal Turkish Journal of Gastroenterology
Volume / Issue Volume 26 · Issue 3
Pages 251-253
History
Published Online May 15, 2015
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Affiliations
Farzaneh Motamed
Department of Pediatrics, Pediatrics Center of Excellence, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
Sanaz Mehrabani
Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran
Maryam Monajemzadeh
Department of Pediatrics, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Mohammad Taghi Haghi Ashtiani
Department of Pediatrics, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Sima Hosseinverdi
Research Center for Immunodeficiencies, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Masoud Houshmand
Department of Medical Genetics, Special Medical Center, Tehran, Iran
Omid Aryani
Department of Medical Genetics, Special Medical Center, Tehran, Iran
Mehri Najafi
Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Fatemeh Farahmand
Department of Pediatrics, Pediatrics Center of Excellence, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
Mohammad Ali Kiani
Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Ahmad Khodadad
Department of Pediatrics, Pediatrics Center of Excellence, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran
Gholam Hossein Fallahi
Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran
Gholamreza Khatami
Department of Pediatrics, Children?s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Nima Rezaei
Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Cite this Article
Motamed, F., Mehrabani, S., Monajemzadeh, M., Ashtiani, M. T. H., Hosseinverdi, S., Houshmand, M., … Rezaei, N. (2015). Low incidence of alpha-1-antitrypsin deficiency in Iranian patients with neonatal cholestasis. Turkish Journal of Gastroenterology, 26(3), 251–253. https://doi.org/10.5152/tjg.2015.6339
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